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Saul Suster

Publications and source records attributed to Saul Suster.

At least 37 records · Page 2Linked to original sources

Sarcoma arising in hyaline-vascular castleman disease of skin and subcutis.

We report on a case of a sarcoma arising in the hyaline-vascular variant of Castleman disease (HVCD) of the skin and subcutis. The patient was a 38-year-old man who clinically presented with a subcutaneous non-fixed cyst-like mass on his right shoulder with an unremarkable prior medical history. Histologic sections showed a biphasic tumor with numerous atretic lymphoid follicles located in the deep dermis and subcutis and a spindle-cell neoplasm mainly situated in the deep subcutis and adjacent soft tissue. The atretic lymphoid component fulfilled the criteria for HVCD, whereas the spindle-cell lesion showed all the criteria for sarcoma including nuclear atypia and frequent mitotic figures. The sarcomatous component was diffusely positive for fascin, nerve growth factor receptor, and CD34 with focal weak reactivity for CD21 and CNA.42. Stains for CD23, CD31, CD35, CD99, ALK-1, SMA, ASMA, desmin, factor XIIIa, AE1-AE3, EMA, bcl-2, S-100, Melan-A, HMB-45, Cam 5.2, and factor VIII were negative in the neoplastic spindle cells. No monoclonal population of lymphocytes was detected and we could not identify EBV or HHV-8 virus by PCR. Electron microscopy of the sarcomatous component showed spindle cells with labyrinth-like invaginations of the nucleus and numerous long, slender, interwoven cytoplasmic processes. The sarcomatous component in this case is most consistent with a poorly differentiated follicular dendritic cell sarcoma based upon the morphologic and ultrastructural findings.

Adult↗

Low-grade serous carcinoma of the ovary metastatic to the anterior mediastinum simulating multilocular thymic cysts: a clinicopathologic and immunohistochemical study of 3 cases.

Three cases of serous borderline tumors of the ovary with areas of serous low-grade carcinoma metastatic to the anterior mediastinum simulating multilocular thymic cysts are presented. The patients are women between the ages of 33 and 50 years. The 3 women had a prior history of primary ovarian neoplasms diagnosed over a period ranging from 3 to 20 years; the 3 patients were in stages IIIA, IIIB, and III. Follow-up radiologic examination revealed the presence of an anterior mediastinal tumor. The 3 patients underwent surgical resection of the mediastinal tumor. Grossly, the mediastinal tumors measured from 7 to 9 cm in greatest diameter and were described as cystic with solid areas. Focal areas of hemorrhage were present, but frank necrosis was not identified. Histologically, all the tumors basically showed similar histopathologic features, namely, those described in multilocular thymic cysts, ie, cystic structures lined by either squamous or low cuboidal epithelium, lymphoid hyperplasia, cholesterol cleft granulomas, and remnants of thymic tissue. In addition, within the cystic structures, there was a neoplastic cellular proliferation with papillary architecture, nuclear atypia, and scattered mitotic figures. Immunohistochemical studies for keratin, MOC31, and CA-125 showed positive staining in tumor cells while placental-like alkaline phosphatase was negative. Two patients remain alive and well after follow-up ranging from 6 to 18 months and 1 patient died of tumor 18 years after initial diagnosis.

Adult↗

Primary synovial sarcomas of the mediastinum: a clinicopathologic, immunohistochemical, and ultrastructural study of 15 cases.

A series of 15 cases of primary mediastinal neoplasms displaying histopathologic, immunohistochemical, and ultrastructural features of synovial sarcoma is presented. The patients' ages ranged from 3 to 83 years, with a male-to-female ratio of 2:1. Nine cases presented as anterior mediastinal masses with chest pain, shortness of breath, and pleural effusion, and 6 cases were in paravertebral location in the posterior mediastinum and presented with neck or back pain and pleural effusion. The tumors measured from 5 to 20 cm in greatest diameter and showed a tan white, soft to rubbery cut surface with areas of hemorrhage and necrosis and foci of gelatinous material. Four cases showed areas of cystic degeneration. In 7 cases, the tumors were well circumscribed; in 6 cases, the tumors grossly invaded the pleura, pericardium, heart, great vessels, chest wall, rib, and vertebra. Histologically, 5 cases displayed a biphasic growth pattern, with well-formed glandular elements admixed with a monotonous spindle cell population. Ten cases were exclusively composed of a monotonous atypical spindle cell proliferation. Immunohistochemical studies showed focal positivity of the tumor cells for cytokeratin and/or epithelial membrane antigen, and strong positivity for vimentin and bcl-2 in the spindle cells in all cases studied (10 of 10). Eight cases also showed focal positivity for CD99. Electron microscopic examination in 5 cases showed oval to spindle tumor cells with closely apposed cell membranes, abundant cytoplasmic intermediate filaments and rough endoplasmic reticulum, and immature desmosome-type cell junctions. Ten patients were treated by complete surgical excision and two by partial excision followed by radiation therapy. In 4 patients, the tumors were inoperable and treated with radiation therapy only. Clinical follow-up was available in 5 patients and showed local recurrence with metastases to lung, lymph nodes, and epidural space from 1 to 3 years in 4 cases and liver metastases and death due to tumor after 6 month in 1 case. Synovial sarcoma should be considered in the differential diagnosis of biphasic and monophasic spindle cell neoplasms of the mediastinum.

Adolescent↗

Mucinous carcinoma of the skin, primary, and secondary: a clinicopathologic study of 63 cases with emphasis on the morphologic spectrum of primary cutaneous forms: homologies with mucinous lesions in the breast.

We present the largest series of mucinous carcinoma involving the skin, describing the histopathologic, immunohistochemical, electron microscopic, and cytogenetic findings. Our aim was fully to characterize the clinicopathologic spectrum and compare it with that seen in the breast. In addition, we wished to reevaluate the differential diagnostic criteria for distinguishing primary mucinous carcinomas from histologically similar neoplasms involving the skin secondarily, and study some aspects of their pathogenesis. We demonstrate that primary cutaneous mucinous carcinomas span a morphologic spectrum compatible to their mammary counterparts. Both pure and mixed types can be delineated morphologically, and some lesions have mucocele-like configurations. Most lesions seem to originate from in situ lesions that may represent, using mammary pathology terminology, ductal hyperplasia, atypical ductal hyperplasia, or ductal carcinoma in situ or a combination of the three. Inverse cell polarity appears to facilitate the progression of the changes similar to lesions in the breast. The presence of an in situ component defines the neoplasm as primary cutaneous, but its absence does not exclude the diagnosis; although for such neoplasms, full clinical assessment is essential. Mammary mucinous carcinoma involving the skin: all patients presented with lesions on chest wall, breast, axilla, and these locations can serve as clue to the breast origin. Microscopically, cutaneous lesions were of both pure and mixed type, and this correlated with the primary in the breast. Dirty necrosis was a constant histologic finding in intestine mucinous carcinomas involving the skin, and this feature may serve as a clue to an intestinal origin.

Adenocarcinoma, Mucinous↗

Pulmonary metastasis from liposarcoma: a clinicopathologic and immunohistochemical study of 24 cases.

A review of the histologic features of pulmonary metastasis and clinical implications of liposarcoma (LS) is given for 24 cases (8 each) of the 3 types of LS: myxoid LS (ML), pleomorphic LS (PL), and dedifferentiated LS (DDL). Most patients were men. Metastatic ML and PL were distributed almost equally among the lung lobes, whereas DDL was more common in the left lower lobe. The metastatic MLs had variable cellularity ranging from singly scattered cells in a hyalinized stroma (treatment-related effect) to hypercellular ML. Most PLs (6/8) were nonlipogenic and resembled an undifferentiated pleomorphic sarcoma. All metastatic DDLs had high-grade histologic features and were predictably nonlipogenic. After pulmonary metastasectomy, 2 patients with ML and 1 with PL were disease-free. The other 6 patients with ML, 7 with PL, and all with DDL had progressive disease. The morphologic features of LS metastatic to the lungs seem diverse but within the spectrum of the histologic type expected from the primary tumor. Overall, the general trend for these LS subsets is progressive disease, metastatic disease for ML and PL with a much shorter interval for PL, and metastatic disease and local recurrence for DDL.

Adolescent↗

Primary parathyroid tumors of the mediastinum: a clinicopathologic and immunohistochemical study of 17 cases.

We describe 17 cases (9 women, 8 men; aged 36 to 72 years) of primary parathyroid tumors occurring primarily in the anterior mediastinum. Clinically and radiologically, all patients had an anterior mediastinal tumor. All patients had metabolic disturbances with calcium and phosphorus. Of 17 patients, 13 had clinical primary hyperparathyroidism, 1 had secondary hyperparathyroidism due to polycystic kidney disease, and 1 had a history of prostatic carcinoma and 1 of chronic obstructive pulmonary disease. In 1 patient, the tumor was found at autopsy. Grossly, the tumors varied in size from 2 to 7 cm in greatest dimension. Histologically, 2 tumors showed features of parathyroid carcinoma, and 15 tumors showed more conventional features of parathyroid adenomas. Six tumors were predominantly oncocytic, 6 were composed predominantly of chief cells, and 3 had mixed cellular composition. Immunohistochemical studies for chromogranin, synaptophysin, low-molecular-weight keratin (CAM 5.2), and parathyroid hormone were performed in 10 cases (8 parathyroid adenomas and 2 parathyroid carcinomas). All the tumors examined showed variable positive reaction for those antibodies. The cases highlight the importance of keeping primary parathyroid tumors in the differential diagnosis of anterior mediastinal tumors.

Adult↗

Malignant mesothelioma: current status of histopathologic diagnosis and molecular profile.

Malignant mesothelioma of the pleura is a relatively rare neoplasm that has been estimated to account for 20 deaths per million males per year in North America and Europe. A causative association has been well established with asbestos exposure. Paradoxically, the incidence of this tumor continues to rise despite public efforts to reduce, contain or eliminate exposure to asbestos fibers over the past few decades. Another paradoxical feature of the disease is that the majority of malignant mesotheliomas represent morphologically low-grade, well-differentiated neoplasms, yet they follow a relentlessly aggressive and virtually uniformly fatal outcome. For this reason, identification of clinical, morphologic, immunohistochemical or molecular genetic parameters is of extremely limited value for prognostication. Surprisingly, for a disease that currently has no known cure, one of the major problems still lies in establishing the correct diagnosis. Diagnosis acquires a particular relevance in light of the medicolegal ramifications of this disease, and diagnosis of malignant mesothelioma is still fraught with difficulties. Despite the advances in modern diagnostic techniques, no specific markers or morphologic features exist that are exclusive to these tumors. Herein, the current status of malignant mesothelioma diagnosis is reviewed, including the possible contributions of modern molecular techniques for their diagnosis.

Animals↗

CDX2 is a useful marker of intestinal-type differentiation: a tissue microarray-based study of 629 tumors from various sites.

CONTEXT: CDX2, a critical nuclear transcription factor for intestinal development, is expressed in intestinal epithelium and adenocarcinomas. OBJECTIVES: To determine if CDX2 is a useful marker for intestinal-type differentiation and to correlate tumor histology with CDX2 staining in colorectal adenocarcinomas. DESIGN: Tissue microarrays from 71 colorectal adenocarcinomas, 31 hepatocellular carcinomas, 47 lung adenocarcinomas, 55 squamous cell carcinomas of the lung, 69 neuroendocrine carcinomas of the lung and 43 of the pancreas, 57 pancreatic adenocarcinomas, and 256 endometrial adenocarcinomas were stained with antibody against CDX2. RESULTS: CDX2 staining was positive in 51 (71.8%) of 71 colorectal cancers, including 38 (74.5%) of 51 well- or moderately differentiated tumors and 13 (65.0%) of 20 high-grade tumors. Of the high-grade tumors, 5 (71.4%) of 7 mucinous, 3 (100%) of 3 signet ring cell, and 5 (50.0%) of 10 poorly differentiated tumors were positive. Other tumors showing occasional CDX2 staining included 1 of 30 well- or moderately differentiated neuroendocrine carcinomas of the lung and 2 of 43 from the pancreas, 1 of 47 lung adenocarcinomas, 3 of 57 pancreatic adenocarcinomas, and 15 of 256 endometrial carcinomas. Hepatocellular, poorly differentiated neuroendocrine carcinoma of the lung and squamous cell carcinomas of the lung were not immunoreactive for CDX2. CONCLUSIONS: CDX2 is a useful marker for intestinal-type differentiation, is rarely seen in tumors from the other sites evaluated, and may be useful in determining the site of origin for some metastatic tumors. However, CDX2 is not a sensitive marker for poorly differentiated colorectal carcinoma.

Adenocarcinoma↗

Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the lung: a clinicopathologic and immunohistochemical study of two cases.

Two cases of primary angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) (ALHE/EH) of the lung are described. Both patients are white, a 60-year-old man and a 27-year-old woman. One patient had a long-standing history of asthma, and the other had symptomatology related to the pulmonary mass. Wedge resections were performed in both cases, and both lesions shared similar histopathologic changes, mainly the presence of a tumor mass with a marked presence of eosinophils in the background, lymphoid hyperplasia, and marked proliferations of small-caliber vessels. Immunohistochemical studies using leukocyte common antigen, L-26, and UCHL-1 nicely stained the lymphoid component of the lesion, and CD31 clearly outlined the vascular component of the process. Clinical follow-up demonstrated that the woman died of status asthmaticus, and the man was alive and well 1 year after surgical resection of the lesion. Both cases highlight the ubiquitous distribution of ALHE/EH and underscore the importance of keeping these lesions in the differential diagnosis of vascular and lymphoid lesions of the lung.

Adult↗

Protein tyrosine phosphatase receptor-type O (PTPRO) exhibits characteristics of a candidate tumor suppressor in human lung cancer.

Previous study in our laboratory demonstrated suppression of the gene for protein tyrosine phosphatase receptor-type O (PTPRO) in primary and established rat hepatomas. The present study showed methylation-mediated silencing of this gene in primary human lung tumors and in several human lung cancer cell lines, one of the characteristics of many tumor-suppressor genes. The reduced expression of PTPRO in the primary lung tumors correlated with the methylation status of its CpG island. Demethylation of the gene by deoxy-5-azacytidine treatment led to its reactivation in a lung cancer line (A549). Overexpression of PTPRO in A549 cells inhibited anchorage-independent growth, delayed reentry of the cells into the cell cycle after release from cell-cycle arrest, and increased susceptibility of the cells to apoptosis. These data have demonstrated the growth-suppressor characteristics of PTPRO that are unique to a classical tumor suppressor.

Base Sequence↗

Mixed epithelial and stromal tumors of the kidney. A report of 22 cases.

Mixed epithelial and stromal tumor of the kidney (MESTK) is a recently described subset of renal neoplasm that tends to occur in middle-aged and older women and is characterized by a distinctive histological appearance. To further characterize this lesion, we report the clinicopathological and immunohistochemical features of 22 additional cases from our institutional files. Grossly, the tumors ranged in size from 1 cm to 14 cm (mean 6.7 cm), were well circumscribed but unencapsulated, and showed a cystic cut surface. The tumors were composed of a spindle cell proliferation that resembled ovarian stroma, as well as an epithelial component lining the cystic structures, which usually consisted of flat to hobnailed cells typical of collecting-duct epithelium. Areas displaying features of Mullerian differentiation were also documented in 6 cases, including epithelium of endometrioid, tubal, clear cell and squamous cell type as well as one case showing an architecture that closely resembled Mullerian adenofibroma and adenosarcoma. Follow-up in 14 patients (average 4.4 years) showed no evidence of recurrence or metastasis. We believe these tumors represent the renal counterpart of similar mixed epithelial and stromal neoplasms occurring in the biliary tract and pancreas, which is also characterized by cystic structures lined by epithelium, admixed with ovarian-type stroma. The differential diagnosis for these tumors includes cystic nephroma and cystic partially differentiated nephroblastoma, which we believe to represent clinically and morphologically distinct entities from MESTK. In particular, the distinction from cystic nephroma in adult male patients is emphasized, and two cases of this entity are included in the study for comparison.

Adult↗

Solitary central myofibroma presenting in the gnathic region.

Solitary myofibroma of adults is an uncommon neoplasm that typically arises in soft tissue and subcutaneous sites in the head and neck, but rarely within bone. When encountered in the jaws, the lesions exhibit clinical and radiographic features suggestive of an odontogenic tumor or cyst as well as several other neoplastic conditions. Tooth mobility, displacement of teeth, and dramatic jaw expansion may be observed. Analogous to other sites of involvement, gnathic myofibromas are biologically indolent and show little or no recurrence following excision. In rare instances, however, the ability to obtain adequate surgical margins by conservative measures may be limited; thus, issues of local control may supercede the importance of biologic potential. We present the radiologic and histopathologic findings in a case of central myofibroma presenting as a large lytic lesion of the mandible. Myofibroma involving the jaw bones represents a unique diagnostic and therapeutic challenge, and accurate diagnosis and management is predicated on careful correlation of radiographic and pathologic findings.

Adult↗

Polypoid leiomyosarcoma of the gallbladder: study of a case associated with adenomyomatous hyperplasia.

A case of polypoid leiomyosarcoma of the gallbladder arising in association with pre-existing adenomyomatous hyperplasia is described. The patient, a 34-year-old woman with symptoms of cholelithiasis, underwent a cholecystectomy for gallstones. The resected specimen showed, in addition to multiple stones, a large, rubbery, broad-based polypoid mass in the fundus. Histologic examination showed a malignant spindle cell proliferation with immunophenotypic features of smooth muscle differentiation. The base of the lesion showed features of adenomyomatous hyperplasia. The possible relationships of this lesion with adenomyomatous hyperplasia and other stromal lesions of the gallbladder are reviewed.

Actins↗

Oral plasmablastic lymphomas in AIDS patients are associated with human herpesvirus 8.

Human herpes virus type 8 (HHV8) has been strongly associated with Kaposi sarcoma, primary effusion lymphoma (PEL), and Castleman's disease. To our knowledge, infection by this virus has not been strongly associated with other hematopathologic malignancies. We examined five oral cavity lymphomas from men with AIDS for HHV8 and HIV-1 by reverse transcriptase in situ polymerase chain reaction, as well as for Epstein-Barr virus (EBV) (EBER-1, -2) using in situ hybridization and HHV8 protein with immunohistochemistry. Four of these tumors were plasmablastic lymphomas; the final case was diffuse large B-cell lymphoma. Most of the neoplastic cells in these five lymphomas contained HHV8 RNA and protein. Further, the four plasmablastic lymphoma cases had tumor cells that contained EBV. HIV-1 RNA was not detected in the tumor cells but was noted in surrounding benign T cells. In comparison, HHV8 RNA was not detected in any of the five oral cavity lymphomas from people who did not have acquired immunosuppression nor in five lymphomas from AIDS patients that were located at a site other than the oral cavity. It is concluded that oral cavity lymphomas from people with AIDS are strongly associated with infection by HHV8 and EBV. Given the poor prognosis of oral cavity lymphomas in immunocompromised patients, therapy directed against the HHV8 and EBV infection may be of therapeutic value.

Adult↗

Cystic adenomatoid tumor of the mediastinum.

A case of adenomatoid tumor presenting as a mass in the anterior mediastinum is described. The patient was a 56-year-old woman with left side chest wall pain who showed a mediastinal mass on chest x-ray and CT scans. Thorough clinical and radiographic examination did not reveal any evidence of tumor elsewhere. At surgery, the tumor was found adjacent to the anterior pericardial reflection. Grossly, the tumor measured 5.5 x 5.5 x 3 cm and showed a homogeneous cut surface with numerous cystic structures that varied from 0.5 to 1.5 cm in greatest diameter. Histologic examination showed numerous cystic spaces lined by flattened or cuboidal epithelial cells. The walls of the cysts showed a proliferation of small canalicular structures lined by round to polygonal epithelioid cells with vacuolated eosinophilic cytoplasm. Immunohistochemical studies showed strong positivity of the epithelioid cells for AE1/AE3 cytokeratin, CK5/CK6, and calretinin. Stains for CK7, CK20, alpha-fetoprotein, CD31, carcinoembryonic antigen, MOC 31, and chromogranin were negative. Electron microscopic examination showed numerous long microvilli on the cell surface and abundant tonofilaments/desmosomal plaques in the tumor cells, characteristic of mesothelial cells. The patient is alive and well and free of recurrence 1 year following surgery. Adenomatoid tumor is a rare neoplasm that should be added in the differential diagnosis of anterior mediastinal masses. Immunohistochemical and ultrastructural studies may be of aid in identifying the characteristic features of mesothelial cells and to avoid mistaking this lesion for more ominous conditions.

Adenomatoid Tumor↗

Castleman disease of the subcutis and underlying skeletal muscle: report of 6 cases.

Castleman disease of the subcutis and skeletal muscle is rare. We have collected a series of 6 cases of extranodal Castleman disease, located in the subcutis and skeletal muscles of the extremities and trunk. Tumors from mediastinal and retroperitoneal soft tissue and those histologically involving peripheral or truncal lymph nodes were excluded. There were four females and two males; ages ranged from 18 to 37 years (mean, 26 years). Locations included thigh (n = 3), anterior chest (n = 2), and upper arm (n = 1). Sizes ranged from 4.0 to 6.0 cm (mean, 5.2 cm). All patients presented with localized disease. One patient had involvement of the mediastinum 1 year prior to the appearance of his soft tissue lesion. None of the patients demonstrated systemic involvement or signs of the POEMS syndrome. Histopathologically, all cases were classified as hyaline-vascular type (HVCD). In 3 cases, follicular dendritic cell dysplasia was observed. In 1 case, the dysplasia was marked. The subcutaneous lesion of 1 patient revealed a maze of capillaries set in a lipomatous background with occasional lymphoid follicles possessing hyalinized lymphocyte-depleted centers. This lesion probably represented incipient HVCD. Molecular biologic studies did not reveal the presence of Epstein-Barr virus or human herpesvirus-8 DNA in the lesional tissue. There also were no monoclonal rearrangements of IgH. Four patients with follow-up included 2 patients with no evidence of disease at 10 and 13 years, respectively, and 2 patients with local recurrence at 2 and 6 months, respectively. In conclusion, soft tissue Castleman disease is a disease of young patients with a female predominance and a propensity to involve the trunk and limbs. It can be of large size and is generally solitary. There may be mild to marked follicular dendritic cell dysplasia. The HVCD predominates in this location. These lesions are usually unassociated with POEMS, Epstein-Barr virus, human herpesvirus-8, or monoclonal rearrangements of IgH.

Adolescent↗

Rhabdomyomatous carcinoma of the thymus.

Myoid cells have been described in the thymus in association with a variety of benign and malignant conditions. The presence of a benign rhabdomyomatous component in a malignant primary thymic epithelial neoplasm, however, is extremely rare. A case of poorly differentiated carcinoma of the thymus arising in the posterior mediastinum containing a prominent rhabdomyomatous component is described. The patient, a 70-year-old woman, was seen for a large posterior mediastinal mass. An open chest biopsy revealed an extensively necrotic tumor composed of islands of atypical cells with vesicular nuclei and prominent nucleoli with numerous mitotic figures admixed with abortive glandular structures. Interspersed with the malignant epithelial components were islands of large cells containing brightly eosinophilic cytoplasm and small, round, excentrically placed nuclei. Immunohistochemical studies showed strong positivity of the epithelial cells for cytokeratin and strong positivity of the myoid cells for pan-actin, desmin, and myogenin. The possible relationship of the rhabdomyomatous component of this tumor with the myoid cells of the thymus is discussed.

Aged↗

Papillary lung carcinoma with prominent "morular" component.

Three cases of primary pulmonary papillary carcinomas with a prominent "morular" component involved 2 women and 1 man (age range, 25-68 years). The patients had symptoms related to the pulmonary mass, including chest pain, cough, and dyspnea. Radiographic evaluation of the thorax revealed the presence of a pulmonary mass. Surgical biopsies were obtained and reported as non-small cell carcinoma. All patients underwent lobectomy. Two tumors were located in the right upper lobe and 1 in the left upper lobe. The tumors were soft, white to tan, without evidence of necrosis or hemorrhage, and 2.5 to 3.5 cm in greatest diameter. The tumors were characterized predominantly by papillary architecture containing numerous "morules" composed of spindle cells without nuclear atypia or mitotic activity. Some morules were floating freely within papillary spaces; others seemed to detach from the papillary structures. Immunohistochemical studies of 2 tumors showed positivity for thyroid transcription factor-1, keratin, and carcinoembryonic antigen and negativity for thyroglobulin. The morules showed positive thyroid transcription factor-1 staining, weak keratin staining, and negative staining for smooth muscle actin, desmin, and HMB-45. These cases highlight an unusual phenomenon, that of primary papillary carcinomas of the lung with a prominent morular component.

Adult↗